Nystagmus, Congenital Treatment & Management

  • Author: Theodore Curtis, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Jan 26, 2010
 

Medical Care

  • Pharmacologically useful agents for patients with nystagmus are primarily GABA agonists or inhibitors of the excitatory neurotransmitter system. The only drug found to be of benefit in adult patients with a history of idiopathic infantile nystagmus is baclofen. This drug has not been approved for use in children. Baclofen has been effective in treating the periodic alternating nystagmus (PAN) subtype.
  • Recent case reports have shown gabapentin to be beneficial in congenital nystagmus, with an improvement in foveation time and vision and a decrease in amplitude and frequency of the nystagmus.
  • McLean et al investigated treatment of congenital nystagmus with memantine or gabapentin in a randomized, double-blind, placebo-controlled trial of 48 patients.[2] Improvement in mean visual acuity was noted in both treatment groups (F=6.2; p=0.004, analysis of variance). Patients with afferent visual defects showed poorer improvements in visual acuity to medication than those with apparently normal visual systems. However, nystagmus intensity (F=7.7; p=0.001) and foveation (F=8.7; p=0.0007) improved in both nystagmus forms as shown on eye movement recordings. Patients in both treatment groups reported vision improvement more often than patients in the placebo group (p=0.03). McLean et al concluded that memantine and gabapentin can improve visual acuity, reduce nystagmus intensity, and improve foveation in congenital nystagmus.
  • Contact lens wear has been noted to diminish infantile nystagmus, presumably by a trigeminal efferent pathway. This may also increase foveation time by avoiding induced spectacle distortion with ocular movement in patients with high degrees of ametropia.
  • Alternative measures, such as biofeedback, acupuncture, or cutaneous head and neck stimulation, have been reported to decrease nystagmus in select (adult) patients with a history of infantile nystagmus.
  • Refractive errors, even low plus, should be prescribed, as this has been shown to improve visual acuity. An optical system with high plus spectacles and high minus contact lenses has also been shown to improve visual acuity in some patients.
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Surgical Care

  • Retrobulbar or intramuscular injection of botulinum toxin (BOTOX®) has been demonstrated to abolish nystagmus temporarily, but patient satisfaction has been poor due to adverse effects, such as ptosis or diplopia, and the need for reinjection.
  • Strabismus surgery is used in patients with certain forms of nystagmus with varying degrees of success.
    • Anderson or Kestenbaum procedures are used to move the eyes into the null zone to diminish an anomalous head position in the setting of idiopathic infantile nystagmus.
    • Recession or simple tenotomy of all 4 horizontal rectus muscles has been advocated; however, preliminary results have been mixed. A pilot study has been completed that showed some improvement, but the definitive study is still pending.[3]
    • Surgery occasionally is used in the treatment of superior oblique myokymia.
    • Hertle et al conducted a prospective interventional case study to determine the effects of early eye muscle surgery on 19 patients younger than 24 months who had infantile nystagmus syndrome.[4] Outcome measures included acuity, head position, strabismic deviation, and eye movement recordings, including waveform types and a nystagmus optimal foveation fraction (NOFF). Improvements were noted in all outcomes measured. Hertle et al concluded that early eye muscle surgery in patients with oculographically infantile nystagmus syndrome improves the oscillation and visual functions. Increases in foveation periods and the NOFF were measureable.
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Consultations

  • Pediatric neurology consultation can be helpful in patients suspected of harboring CNS disease and in evaluating nystagmus for localizing a CNS lesion.
  • Pediatric endocrinology consultation may be useful in patients with optic nerve hypoplasia to assist in evaluation of pituitary function.
  • Pediatric metabolic disease specialists can offer assistance in patients with congenital cataracts or optic atrophy who are thought to have an underlying metabolic abnormality.
  • Pediatric geneticists play an increasing role in the diagnosis and management of patients with nystagmus whose diagnosis is uncertain or who have dysmorphic features.
  • Pediatric neuroradiologists are of considerable value in evaluating the infant brain for abnormalities uncovered with neuroimaging.
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Contributor Information and Disclosures
Author

Theodore Curtis, MD  Assistant Professor, Department of Ophthalmology, University of Colorado; Consulting Staff, Rocky Mountain Lions Eye Institute

Theodore Curtis, MD is a member of the following medical societies: American Academy of Ophthalmology and American Association for Pediatric Ophthalmology and Strabismus

Disclosure: Nothing to disclose.

Coauthor(s)

David T Wheeler, MD  Associate Professor, Departments of Ophthalmology and Pediatrics, Oregon Health & Science University

David T Wheeler, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Bartiss, OD, MD  Medical Director, Ophthalmology, Family Eye Care of the Carolinas

Michael J Bartiss, OD, MD is a member of the following medical societies: American Academy of Ophthalmology, American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus, and North Carolina Medical Society

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

J James Rowsey, MD  Former Director of Corneal Services, St Luke's Cataract and Laser Institute, Florida

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Hackett A, Tarpey PS, Licata A, Cox J, Whibley A, Boyle J, et al. CASK mutations are frequent in males and cause X-linked nystagmus and variable XLMR phenotypes. Eur J Hum Genet. Dec 23 2009;[Medline].

  2. McLean R, Proudlock F, Thomas S, Degg C, Gottlob I. Congenital nystagmus: randomized, controlled, double-masked trial of memantine/gabapentin. Ann Neurol. Feb 2007;61(2):130-8. [Medline].

  3. Hertle RW, Dell'Osso LF, FitzGibbon EJ, Yang D, Mellow SD. Horizontal rectus muscle tenotomy in children with infantile nystagmus syndrome: a pilot study. J AAPOS. Dec 2004;8(6):539-48. [Medline].

  4. Hertle RW, Felius J, Yang D, Kaufman M. Eye muscle surgery for infantile nystagmus syndrome in the first two years of life. Clin Ophthalmol. 2009;3:615-24. [Medline].

  5. Arnoldi KA, Tychsen L. Prevalence of intracranial lesions in children initially diagnosed with disconjugate nystagmus (spasmus nutans) [published erratum appears in J Pediatr Ophthalmol Strabismus 1995 Nov- Dec;32(6):347]. J Pediatr Ophthalmol Strabismus. Sep-Oct 1995;32(5):296-301. [Medline].

  6. Cabot A, Rozet JM, Gerber S, et al. A gene for X-linked idiopathic congenital nystagmus (NYS1) maps to chromosome Xp11.4-p11.3. Am J Hum Genet. Apr 1999;64(4):1141-6. [Medline].

  7. Golubovic S, Marjanovic S, Cvetkovic D, Manic S. The application of hard contact lenses in patients with congenital nystagmus. Fortschr Ophthalmol. 1989;86(5):535-9. [Medline].

  8. Harris C, Berry D. A developmental model of infantile nystagmus. Semin Ophthalmol. 2006;21:63-9. [Medline].

  9. Helveston EM, Ellis FD, Plager DA. Large recession of the horizontal recti for treatment of nystagmus. Ophthalmology. Aug 1991;98(8):1302-5. [Medline].

  10. Hertle RW, Zhu X. Oculographic and clinical characterization of thirty-seven children with anomalous head postures, nystagmus, and strabismus: the basis of a clinical algorithm. J AAPOS. Feb 2000;4(1):25-32. [Medline].

  11. Lennerstrand G, Nordbo OA, Tian S, et al. Treatment of strabismus and nystagmus with botulinum toxin type A. An evaluation of effects and complications. Acta Ophthalmol Scand. Feb 1998;76(1):27-7. [Medline].

  12. Mezawa M, Ishikawa S, Ukai K. Changes in waveform of congenital nystagmus associated with biofeedback treatment. Br J Ophthalmol. Aug 1990;74(8):472-6. [Medline].

  13. Miura K, Hertle RW, FitzGibbon EJ. Effects of tenotomy surgery on congenital nystagmus waveforms in adult patients. Part II. Dynamical systems analysis. Vision Res. Oct 2003;43(22):2357-62. [Medline].

  14. Pratt-Johnson JA. Results of surgery to modify the null-zone position in congenital nystagmus. Can J Ophthalmol. Jun 1991;26(4):219-23. [Medline].

  15. Reinecke RD. Costenbader Lecture. Idiopathic infantile nystagmus: diagnosis and treatment. J AAPOS. Jun 1997;1(2):67-82. [Medline].

  16. Sarvananthan N, Proudlock FA, Choudhuri I. Pharmacologic treatment of congenital nystagmus. Arch Ophthalmol. 2006;124:916-8. [Medline].

  17. Sprunger DT, Wasserman BN, Stidham DB. The relationship between nystagmus and surgical outcome in congenital esotropia. J AAPOS. Feb 2000;4(1):21-4. [Medline].

 
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